Weber christian disease = داء ويبر كريستيان |
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Weber Christian Disease
Clinical Presentation.
Weber-Christian disease now is a nonspecific clinical impression but is a pathologic diagnosis of exclusion. The diagnosis is made much less frequently now than it was in the period from 1950 to 1970, probably due to the greater power of current laboratory testing to reveal lupus erythematosus panniculitis, AAT panniculitis, or histiocytic cytophagic panniculitis in cases that might have been classified as Weber-Christian disease in the past. The classical clinical description is a disease characterized by the appearance of crops of tender nodules and plaques in the subcutaneous fat, usually associated with mild fever. The lower extremities are favored sites, but lesions may occur elsewhere and may ulcerate (Fig. 20-21). The disease occurs mostly in middle-aged women but has been described in all ages and in both sexes. In general, the disease has a good prognosis. However, if internal organ fat necrosis also is present, it may be fatal (80,81,82).
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Histopathology.
The histopathologic appearance itself is not sufficiently specific to exclude other diseases . The classical description of the disease is that it evolves through three stages. In the first phase, there is only erythema and induration clinically. Histologically, acute inflammation is found in the fat lobules with degeneration of fat cells and an infiltrate of neutrophils, lymphocytes, and macrophages. The second phase has an infiltrate discretely localized to the fat lobules, consisting mainly of macrophages, foam cells, and extracellular lipid droplets. In some cases, the lesions perforate the skin surface and discharge a sterile, oily liquid. The third phase
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has depressed and indurated clinical lesions. Fibroblasts, collagen, and scattered lymphocytes and a few plasma cells replace the fat. Vascular changes are mild .
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Systemic lesions occur in some cases of Weber-Christian disease and serve to distinguish this disease from most other types of panniculitis, except for histiocytic cytophagic panniculitis, gamma/delta T-cell lymphoma, and AAT deficiency panniculitis. In autopsy studies, inflammatory changes have been found in perivisceral fat as well as intravisceral fat in the liver, spleen, bone marrow, or near the pleural or peritoneal cavity. A large amount of oily fluid may accumulate in the pleural or peritoneal cavity .
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Pathogenesis. If Weber-Christian disease exists as a distinct disease, the cause is unknown, but circulating immune complexes have been found in some cases .
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Differential Diagnosis.
The histologic appearance of Weber-Christian disease is most distinctive in the second phase, when there is an infiltration by lymphocytes and macrophages between fat cells that is discretely localized to the fat lobules. Erythema nodosum with unusual predominance of neutrophils can resemble the early phase of Weber-Christian disease, except for the localization of erythema nodosum primarily in the septa and WeberChristian mainly in the lobules. Likewise, AA T deficiency can resemble early Weber-Christian disease except that the neutrophilic infiltrate in AAT deficiency usually affects the dermis and septa as well as the lobules. Subcutaneous fat necrosis of pancreatic disease can produce some of these findings but usually can be recognized by the extensive necrosis and deposition of bluish calcium precipitates with fatty acids. Histiocyticcytophagicpanniculitis
has some of the clinical features but histologically has a lymphoid infiltrate with macrophages that
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have ingested sufficient lymphocytes, neutrophils, and eosinophils to be called bean bag cells ).
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Subcutaneous panniculitic T-cell lymphoma can underly a histiocytic cytophagic panniculitic reaction, so lymphoma needs to be considered. After adequate investigation for all these other diseases, very few unexplained cases remain to be classified as Weber-Christian disease.
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